{"id":"https://openalex.org/W2008239986","doi":"https://doi.org/10.1136/jnnp.67.3.290","title":"Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with \"Lorenzo's oil\"","display_name":"Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with \"Lorenzo's oil\"","publication_year":1999,"publication_date":"1999-09-01","ids":{"openalex":"https://openalex.org/W2008239986","doi":"https://doi.org/10.1136/jnnp.67.3.290","mag":"2008239986","pmid":"https://pubmed.ncbi.nlm.nih.gov/10449548","pmcid":"https://www.ncbi.nlm.nih.gov/pmc/articles/1736534"},"language":"en","primary_location":{"id":"doi:10.1136/jnnp.67.3.290","doi":"https://doi.org/10.1136/jnnp.67.3.290","is_oa":true,"landing_page_url":"https://doi.org/10.1136/jnnp.67.3.290","pdf_url":"https://jnnp.bmj.com/content/jnnp/67/3/290.full.pdf","source":{"id":"https://openalex.org/S134773490","display_name":"Journal of Neurology Neurosurgery & Psychiatry","issn_l":"0022-3050","issn":["0022-3050","1468-330X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310319945","host_organization_name":"BMJ","host_organization_lineage":["https://openalex.org/P4310319945"],"host_organization_lineage_names":["BMJ"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Neurology, Neurosurgery & Psychiatry","raw_type":"journal-article"},"volume":null,"type":"article","is_traditional":true,"indexed_in":["crossref","pubmed"],"open_access":{"is_oa":true,"oa_status":"bronze","oa_url":"https://jnnp.bmj.com/content/jnnp/67/3/290.full.pdf","any_repository_has_fulltext":true},"authorships":[{"author_position":"first","author":{"id":"https://openalex.org/A5109996529","display_name":"Bj\u00f6rn M. van Geel","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":true,"raw_author_name":"B M van Geel","raw_affiliation_strings":["Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. b.m.vangeel@amc.uva.nl","Academic Medical Center, University of Amsterdam, Department of Neurology, PO Box 22700, 1100 DE Amsterdam, the Netherlands. Telephone 0031 20 5663842"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. b.m.vangeel@amc.uva.nl","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]},{"raw_affiliation_string":"Academic Medical Center, University of Amsterdam, Department of Neurology, PO Box 22700, 1100 DE Amsterdam, the Netherlands. Telephone 0031 20 5663842","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5034331485","display_name":"Johanna Assies","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"J Assies","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5108522472","display_name":"Elizabeth B. Haverkort","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"E B Haverkort","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5068994001","display_name":"Johannes H.T.M. Koelman","orcid":"https://orcid.org/0009-0008-0611-948X","observed_orcids":["https://orcid.org/0009-0008-0611-948X"]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"J H T M Koelman","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5109251401","display_name":"Bernard Verbeeten Jr.","orcid":null,"observed_orcids":[]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"B Verbeeten","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"middle","author":{"id":"https://openalex.org/A5040743356","display_name":"Ronald J. A. Wanders","orcid":"https://orcid.org/0000-0001-7818-1452","observed_orcids":["https://orcid.org/0000-0001-7818-1452"]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"R J A Wanders","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]},{"author_position":"last","author":{"id":"https://openalex.org/A5025943767","display_name":"Peter G. Barth","orcid":"https://orcid.org/0000-0002-9025-5677","observed_orcids":["https://orcid.org/0000-0002-9025-5677"]},"institutions":[{"id":"https://openalex.org/I2802928900","display_name":"Amsterdam UMC Location University of Amsterdam","ror":"https://ror.org/03t4gr691","country_code":"NL","type":"healthcare","lineage":["https://openalex.org/I2802928900","https://openalex.org/I4210151833"]},{"id":"https://openalex.org/I887064364","display_name":"University of Amsterdam","ror":"https://ror.org/04dkp9463","country_code":"NL","type":"education","lineage":["https://openalex.org/I887064364"]}],"countries":["NL"],"is_corresponding":false,"raw_author_name":"P G Barth","raw_affiliation_strings":["Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands"],"raw_orcid":null,"affiliations":[{"raw_affiliation_string":"Department of Neurology, \n\nbDepartments of Internal Medicine and Psychiatry, \n\ncDepartment of Dietetics, \n\ndDepartment of Neurology, Division of Clinical Neurophysiology, \n\neDepartment of Radiology, \n\nfDepartment of Biochemistry, \n\ngDepartments of Pediatrics and Neurology, Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands","institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"]}]}],"institutions":[],"countries_distinct_count":1,"institutions_distinct_count":2,"corresponding_author_ids":["https://openalex.org/A5109996529"],"corresponding_institution_ids":["https://openalex.org/I2802928900","https://openalex.org/I887064364"],"apc_list":null,"apc_paid":null,"fwci":2.8955,"has_fulltext":true,"cited_by_count":131,"citation_normalized_percentile":{"value":0.91389432,"is_in_top_1_percent":false,"is_in_top_10_percent":true},"cited_by_percentile_year":{"min":88,"max":99},"biblio":{"volume":"67","issue":"3","first_page":"290","last_page":"299"},"is_retracted":false,"is_paratext":false,"is_xpac":false,"primary_topic":{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.9897000193595886,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},"topics":[{"id":"https://openalex.org/T11027","display_name":"Metabolism and Genetic Disorders","score":0.9897000193595886,"subfield":{"id":"https://openalex.org/subfields/1308","display_name":"Clinical Biochemistry"},"field":{"id":"https://openalex.org/fields/13","display_name":"Biochemistry, Genetics and Molecular Biology"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}},{"id":"https://openalex.org/T10945","display_name":"Lysosomal Storage Disorders Research","score":0.00860000029206276,"subfield":{"id":"https://openalex.org/subfields/2716","display_name":"Genetics (clinical)"},"field":{"id":"https://openalex.org/fields/27","display_name":"Medicine"},"domain":{"id":"https://openalex.org/domains/4","display_name":"Health Sciences"}},{"id":"https://openalex.org/T13481","display_name":"Neurological diseases and metabolism","score":0.0003000000142492354,"subfield":{"id":"https://openalex.org/subfields/2808","display_name":"Neurology"},"field":{"id":"https://openalex.org/fields/28","display_name":"Neuroscience"},"domain":{"id":"https://openalex.org/domains/1","display_name":"Life Sciences"}}],"keywords":[{"id":"https://openalex.org/keywords/x-linked-adrenoleukodystrophy","display_name":"X-linked adrenoleukodystrophy","score":0.9430000185966492},{"id":"https://openalex.org/keywords/adrenomyeloneuropathy","display_name":"adrenomyeloneuropathy","score":0.9679999947547913},{"id":"https://openalex.org/keywords/lorenzo-s-oil","display_name":"Lorenzo's oil","score":0.996999979019165},{"id":"https://openalex.org/keywords/very-long-chain-fatty-acids","display_name":"very long chain fatty acids","score":0.8009999990463257},{"id":"https://openalex.org/keywords/hexacosanoic-acid","display_name":"hexacosanoic acid","score":0.9490000009536743},{"id":"https://openalex.org/keywords/peroxisomal-fatty-acid-oxidation","display_name":"peroxisomal fatty acid oxidation","score":0.8700000047683716},{"id":"https://openalex.org/keywords/disease-progression","display_name":"disease progression","score":0.503000020980835},{"id":"https://openalex.org/keywords/neurological-deficit","display_name":"neurological deficit","score":0.46700000762939453},{"id":"https://openalex.org/keywords/adrenal-insufficiency","display_name":"adrenal insufficiency","score":0.8370000123977661},{"id":"https://openalex.org/keywords/hypogonadism","display_name":"hypogonadism","score":0.9599999785423279},{"id":"https://openalex.org/keywords/cerebral-demyelination","display_name":"brain demyelination","score":0.8259999752044678},{"id":"https://openalex.org/keywords/treatment-side-effects","display_name":"treatment side effects","score":0.7099999785423279}],"concepts":[{"id":"https://openalex.org/C2777150068","wikidata":"https://www.wikidata.org/wiki/Q366964","display_name":"Adrenoleukodystrophy","level":4,"score":0.9893463850021362},{"id":"https://openalex.org/C2777910003","wikidata":"https://www.wikidata.org/wiki/Q1707292","display_name":"Asymptomatic","level":2,"score":0.920244574546814},{"id":"https://openalex.org/C2781317408","wikidata":"https://www.wikidata.org/wiki/Q3281322","display_name":"Peroxisomal disorder","level":4,"score":0.6981188654899597},{"id":"https://openalex.org/C71924100","wikidata":"https://www.wikidata.org/wiki/Q11190","display_name":"Medicine","level":0,"score":0.5923267602920532},{"id":"https://openalex.org/C2778129406","wikidata":"https://www.wikidata.org/wiki/Q2507454","display_name":"Adrenal insufficiency","level":2,"score":0.5661166906356812},{"id":"https://openalex.org/C126322002","wikidata":"https://www.wikidata.org/wiki/Q11180","display_name":"Internal medicine","level":1,"score":0.5621140599250793},{"id":"https://openalex.org/C90924648","wikidata":"https://www.wikidata.org/wiki/Q120569","display_name":"Gastroenterology","level":1,"score":0.4609746038913727},{"id":"https://openalex.org/C2909972456","wikidata":"https://www.wikidata.org/wiki/Q2507454","display_name":"Adrenocortical Insufficiency","level":3,"score":0.4483809769153595},{"id":"https://openalex.org/C134018914","wikidata":"https://www.wikidata.org/wiki/Q162606","display_name":"Endocrinology","level":1,"score":0.44159823656082153},{"id":"https://openalex.org/C127078168","wikidata":"https://www.wikidata.org/wiki/Q138639","display_name":"Peroxisome","level":3,"score":0.4393390417098999},{"id":"https://openalex.org/C170493617","wikidata":"https://www.wikidata.org/wiki/Q208467","display_name":"Receptor","level":2,"score":0.0}],"mesh":[{"descriptor_ui":"D000293","descriptor_name":"Adolescent","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D000326","descriptor_name":"Adrenoleukodystrophy","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":true},{"descriptor_ui":"D000328","descriptor_name":"Adult","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000473","qualifier_name":"pathology","is_major_topic":false},{"descriptor_ui":"D001921","descriptor_name":"Brain","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false},{"descriptor_ui":"D002648","descriptor_name":"Child","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D002675","descriptor_name":"Child, Preschool","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004042","descriptor_name":"Dietary Fats, Unsaturated","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D004338","descriptor_name":"Drug Combinations","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D004703","descriptor_name":"Endocrine System","qualifier_ui":"Q000503","qualifier_name":"physiopathology","is_major_topic":false},{"descriptor_ui":"D004883","descriptor_name":"Erucic Acids","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D005071","descriptor_name":"Evoked Potentials","qualifier_ui":"Q000502","qualifier_name":"physiology","is_major_topic":true},{"descriptor_ui":"D005260","descriptor_name":"Female","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D006801","descriptor_name":"Humans","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008040","descriptor_name":"Genetic Linkage","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true},{"descriptor_ui":"D008279","descriptor_name":"Magnetic Resonance Imaging","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D008297","descriptor_name":"Male","qualifier_ui":null,"qualifier_name":null,"is_major_topic":false},{"descriptor_ui":"D014304","descriptor_name":"Triolein","qualifier_ui":"Q000627","qualifier_name":"therapeutic use","is_major_topic":true},{"descriptor_ui":"D014960","descriptor_name":"X Chromosome","qualifier_ui":null,"qualifier_name":null,"is_major_topic":true}],"locations_count":9,"locations":[{"id":"doi:10.1136/jnnp.67.3.290","doi":"https://doi.org/10.1136/jnnp.67.3.290","is_oa":true,"landing_page_url":"https://doi.org/10.1136/jnnp.67.3.290","pdf_url":"https://jnnp.bmj.com/content/jnnp/67/3/290.full.pdf","source":{"id":"https://openalex.org/S134773490","display_name":"Journal of Neurology Neurosurgery & Psychiatry","issn_l":"0022-3050","issn":["0022-3050","1468-330X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310319945","host_organization_name":"BMJ","host_organization_lineage":["https://openalex.org/P4310319945"],"host_organization_lineage_names":["BMJ"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Neurology, Neurosurgery & Psychiatry","raw_type":"journal-article"},{"id":"pmh:oai:pubmedcentral.nih.gov:1736534","doi":null,"is_oa":true,"landing_page_url":"https://www.ncbi.nlm.nih.gov/pmc/articles/1736534","pdf_url":null,"source":{"id":"https://openalex.org/S2764455111","display_name":"PubMed Central","issn_l":null,"issn":null,"is_oa":true,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"J Neurol Neurosurg Psychiatry","raw_type":"Text"},{"id":"pmid:10449548","doi":null,"is_oa":false,"landing_page_url":"https://pubmed.ncbi.nlm.nih.gov/10449548","pdf_url":null,"source":{"id":"https://openalex.org/S4306525036","display_name":"PubMed","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1299303238","host_organization_name":"National Institutes of Health","host_organization_lineage":["https://openalex.org/I1299303238"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of neurology, neurosurgery, and psychiatry","raw_type":"Clinical Trial"},{"id":"pmh:oai:dare.uva.nl:openaire/9f848ff6-9567-41cf-9caa-83463c4c4840","doi":null,"is_oa":true,"landing_page_url":"https://handle.uba.uva.nl/personal/pure/en/publications/progression-of-abnormalities-in-adrenomyeloneuropathy-and-neurologically-asymptomatic-xlinked-adrenoleukodystrophy-despite-treatment-with-lorenzos-oil(9f848ff6-9567-41cf-9caa-83463c4c4840).html","pdf_url":null,"source":{"id":"https://openalex.org/S4306400088","display_name":"UvA-DARE (University of Amsterdam)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I887064364","host_organization_name":"University of Amsterdam","host_organization_lineage":["https://openalex.org/I887064364"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"van Geel, B M, Assies, J, Haverkort, E B, Koelman, J H T M, Verbeeten, B, Wanders, R J A, Barth, P G 1999, 'Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with \"Lorenzo's oil\"', Journal of Neurology, Neurosurgery and Psychiatry, vol. 67, no. 3, pp. 290-299. https://doi.org/10.1136/jnnp.67.3.290","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:uvapub:oai:dare.uva.nl:publications/344fed4f-3745-4dd2-a210-120b830f24e4","doi":null,"is_oa":true,"landing_page_url":"https://dare.uva.nl/personal/pure/en/publications/progression-of-abnormalities-in-adrenomyeloneuropathy-and-neurologically-asymptomatic-xlinked-adrenoleukodystrophy-despite-treatment-with-lorenzos-oil(344fed4f-3745-4dd2-a210-120b830f24e4).html","pdf_url":"https://dare.uva.nl/personal/pure/en/publications/progression-of-abnormalities-in-adrenomyeloneuropathy-and-neurologically-asymptomatic-xlinked-adrenoleukodystrophy-despite-treatment-with-lorenzos-oil(344fed4f-3745-4dd2-a210-120b830f24e4).html","source":{"id":"https://openalex.org/S4306401843","display_name":"Data Archiving and Networked Services (DANS)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1322597698","host_organization_name":"Royal Netherlands Academy of Arts and Sciences","host_organization_lineage":["https://openalex.org/I1322597698"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Journal of Neurology, Neurosurgery and Psychiatry, 67, 290 - 299. BMJ Publishing Group","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:CiteSeerX.psu:10.1.1.893.4975","doi":null,"is_oa":false,"landing_page_url":"http://citeseerx.ist.psu.edu/viewdoc/summary?doi=10.1.1.893.4975","pdf_url":null,"source":null,"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"http://dare.uva.nl/document/2/4470/","raw_type":"text"},{"id":"pmh:amcpub:oai:pure.amc.nl:publications/f4626a4b-6bc4-4ce6-afe4-4ce627841bc0","doi":null,"is_oa":false,"landing_page_url":"https://pure.amc.nl/en/publications/progression-of-abnormalities-in-adrenomyeloneuropathy-and-neurologically-asymptomatic-xlinked-adrenoleukodystrophy-despite-treatment-with-lorenzos-oil(f4626a4b-6bc4-4ce6-afe4-4ce627841bc0).html","pdf_url":null,"source":{"id":"https://openalex.org/S4306401843","display_name":"Data Archiving and Networked Services (DANS)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1322597698","host_organization_name":"Royal Netherlands Academy of Arts and Sciences","host_organization_lineage":["https://openalex.org/I1322597698"],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Journal of neurology, neurosurgery, and psychiatry, 67(3), 290 - 299. BMJ Publishing Group","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:oai:pure.amsterdamumc.nl:openaire_cris_publications/8aba2e54-4500-400b-99d5-750496aa2526","doi":null,"is_oa":false,"landing_page_url":"https://pure.amsterdamumc.nl/en/publications/8aba2e54-4500-400b-99d5-750496aa2526","pdf_url":null,"source":{"id":"https://openalex.org/S7407055222","display_name":"Pure Amsterdam UMC","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":null,"host_organization_name":null,"host_organization_lineage":[],"host_organization_lineage_names":[],"type":"repository"},"license":null,"license_id":null,"version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"van Geel, B M, Assies, J, Haverkort, E B, Koelman, J H, Verbeeten, B, Wanders, R J & Barth, P G 1999, 'Progression of abnormalities in adrenomyeloneuropathy and neurologically asymptomatic X-linked adrenoleukodystrophy despite treatment with \"Lorenzo's oil\"', Journal of neurology, neurosurgery, and psychiatry, vol. 67, no. 3, pp. 290-299. https://doi.org/10.1136/jnnp.67.3.290","raw_type":"info:eu-repo/semantics/article"},{"id":"pmh:uvapub:oai:dare.uva.nl:publications/9f848ff6-9567-41cf-9caa-83463c4c4840","doi":null,"is_oa":true,"landing_page_url":"https://dare.uva.nl/personal/pure/en/publications/progression-of-abnormalities-in-adrenomyeloneuropathy-and-neurologically-asymptomatic-xlinked-adrenoleukodydtrophy-despite-treatment-with-lozenzos-oil(9f848ff6-9567-41cf-9caa-83463c4c4840).html","pdf_url":null,"source":{"id":"https://openalex.org/S4306401843","display_name":"Data Archiving and Networked Services (DANS)","issn_l":null,"issn":null,"is_oa":false,"is_in_doaj":false,"is_core":false,"listed_in":[],"host_organization":"https://openalex.org/I1322597698","host_organization_name":"Royal Netherlands Academy of Arts and Sciences","host_organization_lineage":["https://openalex.org/I1322597698"],"host_organization_lineage_names":[],"type":"repository"},"license":"other-oa","license_id":"https://openalex.org/licenses/other-oa","version":"submittedVersion","is_accepted":false,"is_published":false,"raw_source_name":"Journal of Neurology, Neurosurgery and Psychiatry, 67(3), 290 - 299. BMJ Publishing Group","raw_type":"info:eu-repo/semantics/article"}],"best_oa_location":{"id":"doi:10.1136/jnnp.67.3.290","doi":"https://doi.org/10.1136/jnnp.67.3.290","is_oa":true,"landing_page_url":"https://doi.org/10.1136/jnnp.67.3.290","pdf_url":"https://jnnp.bmj.com/content/jnnp/67/3/290.full.pdf","source":{"id":"https://openalex.org/S134773490","display_name":"Journal of Neurology Neurosurgery & Psychiatry","issn_l":"0022-3050","issn":["0022-3050","1468-330X"],"is_oa":false,"is_in_doaj":false,"is_core":true,"listed_in":["anvur-scientific","cwts-core","dongbi-a","doyens","jufo-3","ki-jl-2","medline","norway-2","poland-140","russia-white-list-1","vabb-shw"],"host_organization":"https://openalex.org/P4310319945","host_organization_name":"BMJ","host_organization_lineage":["https://openalex.org/P4310319945"],"host_organization_lineage_names":["BMJ"],"type":"journal"},"license":null,"license_id":null,"version":"publishedVersion","is_accepted":true,"is_published":true,"raw_source_name":"Journal of Neurology, Neurosurgery & Psychiatry","raw_type":"journal-article"},"sustainable_development_goals":[{"score":1.0,"id":"https://metadata.un.org/sdg/3","display_name":"Good health and well-being"}],"study_designs":[{"id":"https://openalex.org/study-designs/observational-study","display_name":"Observational Study"}],"awards":[],"funders":[{"id":"https://openalex.org/F4320321008","display_name":"Universiteit van Amsterdam","ror":"https://ror.org/04dkp9463"}],"has_content":{"pdf":true,"grobid_xml":true},"content_urls":{"pdf":"https://content.openalex.org/works/W2008239986.pdf","grobid_xml":"https://content.openalex.org/works/W2008239986.grobid-xml"},"referenced_works_count":57,"referenced_works":["https://openalex.org/W137925544","https://openalex.org/W189903418","https://openalex.org/W242769242","https://openalex.org/W312269775","https://openalex.org/W1483242733","https://openalex.org/W1542881762","https://openalex.org/W1966355534","https://openalex.org/W1969661146","https://openalex.org/W1978903497","https://openalex.org/W1990589349","https://openalex.org/W1990906452","https://openalex.org/W1995525187","https://openalex.org/W2000006102","https://openalex.org/W2003069142","https://openalex.org/W2003644239","https://openalex.org/W2004164378","https://openalex.org/W2004346519","https://openalex.org/W2005530833","https://openalex.org/W2010687728","https://openalex.org/W2017234490","https://openalex.org/W2018313364","https://openalex.org/W2020287578","https://openalex.org/W2020509659","https://openalex.org/W2024607954","https://openalex.org/W2031484864","https://openalex.org/W2033561631","https://openalex.org/W2035026471","https://openalex.org/W2035260979","https://openalex.org/W2035851563","https://openalex.org/W2037337148","https://openalex.org/W2048833214","https://openalex.org/W2064716240","https://openalex.org/W2077891256","https://openalex.org/W2078576948","https://openalex.org/W2084400497","https://openalex.org/W2089338876","https://openalex.org/W2101153945","https://openalex.org/W2106485907","https://openalex.org/W2107786477","https://openalex.org/W2112165124","https://openalex.org/W2123836280","https://openalex.org/W2135621241","https://openalex.org/W2137919831","https://openalex.org/W2143402754","https://openalex.org/W2147636525","https://openalex.org/W2159972914","https://openalex.org/W2162373952","https://openalex.org/W2164071022","https://openalex.org/W2169422711","https://openalex.org/W2171074759","https://openalex.org/W2222090074","https://openalex.org/W2320665909","https://openalex.org/W2340133774","https://openalex.org/W2416728968","https://openalex.org/W4250051213","https://openalex.org/W4250343703","https://openalex.org/W6655815194"],"related_works":["https://openalex.org/W2149746988","https://openalex.org/W2104646605","https://openalex.org/W2087755698","https://openalex.org/W2165938640","https://openalex.org/W2053754943","https://openalex.org/W7170198270","https://openalex.org/W4292121698","https://openalex.org/W2164291221","https://openalex.org/W2416092981","https://openalex.org/W2145948329"],"abstract_inverted_index":{"OBJECTIVES:":[0],"X-linked":[1],"adrenoleukodystrophy":[2],"(X-ALD)":[3],"is":[4,296],"an":[5],"inherited":[6],"disorder":[7],"of":[8,18,36,54,61,65,72,102,284],"peroxisomal":[9],"fatty":[10,23],"acid":[11,28],"oxidation,":[12],"biochemically":[13],"characterised":[14],"by":[15],"the":[16,51,58,69,114,141,152,168,232,285,288],"accumulation":[17],"saturated":[19],"very":[20],"long":[21],"chain":[22],"acids":[24],"(VLCFAs),":[25],"particularly":[26],"hexacosanoic":[27],"(C26:0).":[29],"Dietary":[30],"treatment":[31,271],"with":[32,90,151,171,182,200,207,213,272,308],"a":[33,99,257],"4:1":[34],"mixture":[35],"glyceroltrioleate":[37],"and":[38,121,179,187,202,210,223,251,263],"glyceroltrierucate":[39],"(\"Lorenzo's":[40],"oil\")":[41],"normalises":[42],"plasma":[43,129],"VLCFA":[44],"concentrations,":[45],"but":[46],"neither":[47,275],"ameliorates":[48],"nor":[49,281],"arrests":[50],"rapid":[52],"progression":[53,283],"neurological":[55,172,277,313],"symptoms":[56],"in":[57,68,136,140,167,197,204,242,260],"cerebral":[59,185,226],"variants":[60],"X-ALD.":[62],"The":[63,128,267],"efficacy":[64],"\"Lorenzo's":[66,91,273,299],"oil\"":[67,92,274,300],"milder":[70],"phenotypes":[71],"X-ALD":[73,309],"was":[74],"assessed,":[75],"as":[76,149],"this":[77],"has":[78],"been":[79],"much":[80],"less":[81],"investigated.":[82],"METHODS:":[83],"Twenty":[84],"two":[85,211],"patients":[86,138,170,212,307],"who":[87,310],"were":[88,107,123,237],"treated":[89],"for":[93,98],"at":[94],"least":[95],"12":[96],"months":[97],"median":[100],"period":[101],"2.5":[103],"(range":[104],"1.0-6.0)":[105],"years":[106],"studied.":[108],"Two":[109],"had":[110],"asymptomatic":[111,208],"ALD,":[112],"four":[113],"\"Addison":[115,176,189],"only\"":[116,177,190],"variant,":[117],"13":[118],"adrenomyeloneuropathy":[119],"(AMN),":[120],"three":[122,142],"symptomatic":[124],"female":[125],"carriers.":[126],"RESULTS:":[127],"C26:0":[130],"concentration":[131,262],"normalised":[132,135],"or":[133,278],"near":[134],"19":[137],"(86%),":[139],"others":[143],"it":[144,295],"decreased":[145],"significantly.":[146],"Nevertheless,":[147],"disability":[148,154],"measured":[150],"extended":[153],"status":[155],"scale":[156],"score":[157],"increased":[158],"mildly":[159],"(0.5":[160],"(95%":[161,164],"confidence":[162],"interval":[163],"CI)":[165],"0.25-1.0))":[166],"16":[169],"symptoms.":[173],"Furthermore,":[174,287],"one":[175,180,198,205],"patient":[178,181,191,199,206],"AMN":[183],"developed":[184,192],"demyelination,":[186],"another":[188],"AMN.":[193,214],"Adrenocortical":[194],"insufficiency":[195],"evolved":[196],"AMN,":[201],"hypogonadism":[203],"ALD":[209],"Nerve":[215],"conduction,":[216],"evoked":[217],"potential":[218],"studies":[219],"(SEP,":[220],"BAEP,":[221],"VEP),":[222],"abnormalities":[224],"on":[225],"MRI":[227],"did":[228],"not":[229,302],"improve.":[230],"On":[231],"other":[233],"hand,":[234],"side":[235],"effects":[236],"often":[238,290],"noted-namely,":[239],"mild":[240,258],"increases":[241],"liver":[243],"enzymes":[244],"(55%),":[245,247],"thrombocytopenia":[246],"gastrointestinal":[248],"complaints":[249],"(14%),":[250],"gingivitis":[252],"(14%).":[253],"We":[254],"also":[255],"found":[256],"decrease":[259],"haemoglobin":[261],"leucocyte":[264],"count.":[265],"CONCLUSIONS:":[266],"data":[268],"suggest":[269],"that":[270,298],"improved":[276],"endocrine":[279],"function":[280],"arrested":[282],"disease.":[286],"oil":[289],"induced":[291],"adverse":[292],"effects.":[293],"Therefore,":[294],"advocated":[297],"should":[301],"be":[303],"prescribed":[304],"routinely":[305],"to":[306],"already":[311],"have":[312],"deficits.":[314]},"counts_by_year":[{"year":2026,"cited_by_count":2},{"year":2025,"cited_by_count":2},{"year":2024,"cited_by_count":7},{"year":2023,"cited_by_count":1},{"year":2022,"cited_by_count":9},{"year":2021,"cited_by_count":3},{"year":2020,"cited_by_count":2},{"year":2019,"cited_by_count":4},{"year":2018,"cited_by_count":2},{"year":2017,"cited_by_count":3},{"year":2016,"cited_by_count":5},{"year":2015,"cited_by_count":2},{"year":2014,"cited_by_count":7},{"year":2013,"cited_by_count":7},{"year":2012,"cited_by_count":3}],"updated_date":"2026-11-04T10:54:05.112534","created_date":"2016-06-24T00:00:00"}